Raynaud syndrome

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Raynaud syndrome
Other namesRaynaud's, Raynaud's disease, Raynaud's phenomenon, Raynaud's syndrome [1]
Raynaud syndrome on female airman's hand.jpg
The hand of a person with Raynaud syndrome during an attack.
Pronunciation
Specialty Rheumatology
Symptoms An affected part turning white, then blue, then red, burning [2]
Complications skin sores, gangrene [2]
Usual onset15–30 year old, typically females [3] [4]
DurationUp to several hours per episode [2]
Risk factors Cold, emotional stress [2]
Diagnostic method Based on the symptoms [3]
Differential diagnosis Causalgia, erythromelalgia [5]
TreatmentAvoiding cold, calcium channel blockers, iloprost [3]
Frequency4% of people [3]
Named after Maurice Raynaud

Raynaud syndrome, also known as Raynaud's phenomenon, is a medical condition in which the spasm of small arteries causes episodes of reduced blood flow to end arterioles. [1] Typically the fingers, and, less commonly, the toes, are involved. [1] Rarely, the nose, ears, nipples, or lips are affected. [1] The episodes classically result in the affected part turning white and then blue. [2] Often, numbness or pain occurs. [2] As blood flow returns, the area turns red and burns. [2] The episodes typically last minutes but can last several hours. [2] The condition is named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862. [6]

Contents

Episodes are typically triggered by cold or emotional stress. [2] Primary Raynaud's is idiopathic (spontaneous and of unknown cause) and not correlated with another disease. Secondary Raynaud's is diagnosed given the presence of an underlying condition and is associated with an older age of onset. [3] In comparison to primary Raynaud's, episodes are more likely to be painful, asymmetric and progress to digital ulcerations. [7] Secondary Raynaud's can occur due to a connective-tissue disorder such as scleroderma or lupus, injuries to the hands, prolonged vibration, smoking, thyroid problems, and certain medications, such as birth control pills and stimulants. [8] Diagnosis is typically based on the symptoms. [3]

The primary treatment is avoiding the cold. [3] Other measures include the discontinuation of nicotine or stimulant use. [3] Medications for treatment of cases that do not improve include calcium channel blockers and iloprost. [3] There is little evidence that alternative medicine is helpful. [3] Severe disease may in rare cases lead to complications, specifically skin sores or gangrene. [2]

About 4% of people have the condition. [3] Onset of the primary form is typically between ages 15 and 30 and occurs more frequently in females. [3] [4] The secondary form usually affects older people. [4] Both forms are more common in cold climates. [4]

Signs and symptoms

Raynaud's affecting all five fingers Raynauld.jpg
Raynaud's affecting all five fingers
Bluish coloration Hand with Raynaud's Syndrome.jpg
Bluish coloration

The condition can cause localized pain, discoloration (paleness), and sensations of cold and/or numbness.

When exposed to cold temperatures, the blood supply to the fingers or toes, and in some cases the nose or earlobes, is markedly reduced; the skin turns pale or white (called pallor) and becomes cold and numb. These events are episodic, and when the episode subsides or the area is warmed, the blood flow returns, and the skin color first turns red (rubor), and then back to normal, often accompanied by swelling, tingling, and a painful "pins and needles" sensation. All three color changes are observed in classic Raynaud's yet not all patients see all of the aforementioned color changes in all episodes, especially in milder cases of the condition. The red flush is due to reactive hyperemia of the areas deprived of blood flow.

In pregnancy, this sign normally disappears due to increased surface blood flow. Raynaud's has occurred in breastfeeding mothers, causing nipples to turn white and painful. [9]

Nipple blanching, or vasospasm of the nipple, can cause pain and difficulty breastfeeding. Nipple-blanching.jpg
Nipple blanching, or vasospasm of the nipple, can cause pain and difficulty breastfeeding.

Causes

Primary

Raynaud's disease, or primary Raynaud's, is diagnosed if the symptoms are idiopathic, that is, if they occur by themselves and not in association with other diseases. Some refer to primary Raynaud's disease as "being allergic to coldness". It often develops in young women in their teens and early adulthood. Primary Raynaud's is thought to be at least partly hereditary, although specific genes have not yet been identified. [10]

Smoking increases frequency and intensity of attacks, and a hormonal component exists. Caffeine, estrogen, and nonselective beta-blockers are often listed as aggravating factors, but evidence that they should be avoided is not solid. [11]

Secondary

Raynaud's phenomenon, or secondary Raynaud's, occurs secondary to a wide variety of other conditions.

Secondary Raynaud's has a number of associations: [12]

Raynaud's can precede these other diseases by many years, making it the first presenting symptom. This may be the case in the CREST syndrome, of which Raynaud's is a part.[ citation needed ]

Patients with secondary Raynaud's can also have symptoms related to their underlying diseases. Raynaud's phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.[ citation needed ]

When Raynaud's phenomenon is limited to one hand or one foot, it is referred to as unilateral Raynaud's. This is an uncommon form, and it is always secondary to local or regional vascular disease. It commonly progresses within several years to affect other limbs as the vascular disease progresses. [17]

Mechanism

Three main changes are seen in the mechanism of Raynaud's phenomenon which are reduced blood flow, blood vessel constriction and neurogenic, inflammatory, and immune responses. It is induced by mental stress and cold atmosphere. In all cases, the primary cause is an underlying hyperactivation of the sympathetic nervous system. Although, with different types, the exact pathophysiology differs. In primary type there is increase in sensitivity due to reasons mentioned above resulting in vasoconstriction. In secondary type, normal activity of blood vessel is disrupted due to the same reasons mentioned above causing vasoconstriction which leads to ischemia and tissue death. [18]

Underlying mechanism Raynaud's Disease.jpg
Underlying mechanism

Diagnosis

Thermogram depicting a Raynaud's hand (top) and a healthy hand (bottom): Red indicates warm areas whilst green indicates cool areas. A thermal image demonstrating the loss of heat in a Reynaud's sufferer.jpg
Thermogram depicting a Raynaud's hand (top) and a healthy hand (bottom): Red indicates warm areas whilst green indicates cool areas.
Consensus diagnostic criteria Consensus Diagnostic Criteria Raynaud's Phenomenon.pdf
Consensus diagnostic criteria

Distinguishing Raynaud's disease (primary Raynaud's) from Raynaud's phenomenon (secondary Raynaud's) is important. Looking for signs of arthritis or vasculitis, as well as a number of laboratory tests, may separate them. Nail fold capillary examination or "capillaroscopy" is one of the most sensitive methods to diagnose RS with connective tissue disorders, i.e. distinguish a secondary from a primary form objectively. [19]

If suspected to be secondary to systemic sclerosis, one tool which may help aid in the prediction of systemic sclerosis is thermography. [20]

A careful medical history will seek to identify or exclude possible secondary causes.

To aid in the diagnosis of Raynaud's phenomenon, multiple sets of diagnostic criteria have been proposed. [21] [22] [23] [24] Table 1 below provides a summary of these various diagnostic criteria. [25]

Recently, International Consensus Criteria were developed for the diagnosis of primary Raynaud's phenomenon by a panel of experts in the fields of rheumatology and dermatology. [25]

Management

Secondary Raynaud's is managed primarily by treating the underlying cause, and as primary Raynaud's, avoiding triggers, such as cold, emotional and environmental stress, vibrations and repetitive motions, and avoiding smoking (including passive smoking) and sympathomimetic drugs. [26]

Medications

Medications can be helpful for moderate or severe disease.

Surgery

Alternative medicine

Evidence does not support the use of alternative medicine, including acupuncture and laser therapy. [3]

Prognosis

The prognosis of primary Raynaud syndrome is often very favorable, with no mortality and little morbidity overall. In some very rare cases, gangrene has been known to develop. The prognosis of secondary Raynaud is related to the course of the underlying disease, and how effective blood flow-restoring maneuvers are. [38]

Related Research Articles

<span class="mw-page-title-main">Systemic scleroderma</span> Accumulation of collagen in the skin and internal organs

Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above, the elbows and knees with or without involvement of the face. The diffuse form also affects the skin above the elbows and knees and can also spread to the torso. Visceral organs, including the kidneys, heart, lungs, and gastrointestinal tract can also be affected by the fibrotic process. Prognosis is determined by the form of the disease and the extent of visceral involvement. Patients with limited systemic sclerosis have a better prognosis than those with the diffuse form. Death is most often caused by lung, heart, and kidney involvement. The risk of cancer is increased slightly.

Rheumatology is a branch of medicine devoted to the diagnosis and management of disorders whose common feature is inflammation in the bones, muscles, joints, and internal organs. Rheumatology covers more than 100 different complex diseases, collectively known as rheumatic diseases, which includes many forms of arthritis as well as lupus and Sjögren's syndrome. Doctors who have undergone formal training in rheumatology are called rheumatologists.

Microvascular angina (MVA), previously known as cardiac syndrome X, also known as coronary microvascular dysfunction(CMD) or microvascular coronary disease is a type of angina (chest pain) with signs associated with decreased blood flow to heart tissue but with normal coronary arteries.

<span class="mw-page-title-main">Erythromelalgia</span> Inflammation due to periodic blood vessel blockage

Erythromelalgia or Mitchell's disease is a rare vascular peripheral pain disorder in which blood vessels, usually in the lower extremities or hands, are episodically blocked, then become hyperemic and inflamed. There is severe burning pain and skin redness. The attacks are periodic and are commonly triggered by heat, pressure, mild activity, exertion, insomnia or stress. Erythromelalgia may occur either as a primary or secondary disorder. Secondary erythromelalgia can result from small fiber peripheral neuropathy of any cause, polycythemia vera, essential thrombocythemia, hypercholesterolemia, mushroom or mercury poisoning, and some autoimmune disorders. Primary erythromelalgia is caused by mutation of the voltage-gated sodium channel α-subunit gene SCN9A.

<span class="mw-page-title-main">CREST syndrome</span> Connective tissue disorder

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

Eosinophilic fasciitis, also known as Shulman's syndrome, is an inflammatory disease that affects the fascia, other connective tissues, surrounding muscles, blood vessels and nerves. Unlike other forms of fasciitis, eosinophilic fasciitis is typically self-limited and confined to the arms and legs, although it can require treatment with corticosteroids, and some cases are associated with aplastic anemia.

<span class="mw-page-title-main">Variant angina</span> Cardiac chest pain at any time, not just periods of exertion

Variant angina, also known as Prinzmetal angina,vasospastic angina, angina inversa, coronary vessel spasm, or coronary artery vasospasm, is a syndrome typically consisting of angina. Variant angina differs from stable angina in that it commonly occurs in individuals who are at rest or even asleep, whereas stable angina is generally triggered by exertion or intense exercise. Variant angina is caused by vasospasm, a narrowing of the coronary arteries due to contraction of the heart's smooth muscle tissue in the vessel walls. In comparison, stable angina is caused by the permanent occlusion of these vessels by atherosclerosis, which is the buildup of fatty plaque and hardening of the arteries.

Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below 30 °C (86 °F), directed against red blood cells, causing them to agglutinate and undergo lysis. It is a form of autoimmune hemolytic anemia, specifically one in which antibodies bind red blood cells only at low body temperatures, typically 28–31 °C.

<span class="mw-page-title-main">Vascular disease</span> Medical condition

Vascular disease is a class of diseases of the vessels of the circulatory system in the body, including blood vessels – the arteries and veins, and the lymphatic vessels. Vascular disease is a subgroup of cardiovascular disease. Disorders in this vast network of blood and lymph vessels can cause a range of health problems that can sometimes become severe, and fatal. Coronary heart disease for example, is the leading cause of death for men and women in the United States.

Mixed connective tissue disease (MCTD) is a systemic autoimmune disease that shares characteristics with at least two other systemic autoimmune diseases, including systemic sclerosis (Ssc), systemic lupus erythematosus (SLE), polymyositis/dermatomyositis (PM/DM), and rheumatoid arthritis. The idea behind the "mixed" disease is that this specific autoantibody is also present in other autoimmune diseases such as systemic lupus erythematosus, polymyositis, scleroderma, etc. MCTD was characterized as an individual disease in 1972 by Sharp et al., and the term was introduced by Leroy in 1980.

<span class="mw-page-title-main">Acrocyanosis</span> Persistent blue or cyanotic discoloration of the extremities

Acrocyanosis is persistent blue or cyanotic discoloration of the extremities, most commonly occurring in the hands, although it also occurs in the feet and distal parts of the face. Although described over 100 years ago and not uncommon in practice, the nature of this phenomenon is still uncertain. The very term "acrocyanosis" is often applied inappropriately in cases when blue discoloration of the hands, feet, or parts of the face is noted. The principal (primary) form of acrocyanosis is that of a benign cosmetic condition, sometimes caused by a relatively benign neurohormonal disorder. Regardless of its cause, the benign form typically does not require medical treatment. A medical emergency would ensue if the extremities experience prolonged periods of exposure to the cold, particularly in children and patients with poor general health. However, frostbite differs from acrocyanosis because pain often accompanies the former condition, while the latter is very rarely associated with pain. There are also a number of other conditions that affect hands, feet, and parts of the face with associated skin color changes that need to be differentiated from acrocyanosis: Raynaud phenomenon, pernio, acrorygosis, erythromelalgia, and blue finger syndrome. The diagnosis may be challenging in some cases, especially when these syndromes co-exist.

Scleromyositis, is an autoimmune disease. People with scleromyositis have symptoms of both systemic scleroderma and either polymyositis or dermatomyositis, and is therefore considered an overlap syndrome. Although it is a rare disease, it is one of the more common overlap syndromes seen in scleroderma patients, together with MCTD and Antisynthetase syndrome. Autoantibodies often found in these patients are the anti-PM/Scl (anti-exosome) antibodies.

<span class="mw-page-title-main">Alpha blocker</span> Class of pharmacological agents

Alpha blockers, also known as α-blockers or α-adrenoreceptor antagonists, are a class of pharmacological agents that act as antagonists on α-adrenergic receptors (α-adrenoceptors).

<span class="mw-page-title-main">Scleroderma</span> Group of autoimmune diseases resulting in abnormal growth of connective tissue

Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure. One form of the condition, known as CREST syndrome, classically results in calcium deposits, Raynaud's syndrome, esophageal problems, thickening of the skin of the fingers and toes, and areas of small, dilated blood vessels.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which a person's blood plasma is allowed to cool substantially, causing the (reversible) precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins.

Undifferentiated connective tissue disease (UCTD) is a disease in which the connective tissues are targeted by the immune system. It is a serological and clinical manifestation of an autoimmune disease. When there is proof of an autoimmune disease, but the disease does not correspond to any specific autoimmune disease, it will be diagnosed as UCTD. This is also the case of major rheumatic diseases whose early phase was defined by LeRoy et al in 1980 as undifferentiated connective tissue disease.

<span class="mw-page-title-main">Antisynthetase syndrome</span> Medical condition

Antisynthetase syndrome (ASS) is a multisystematic autoimmune disease associated with inflammatory myositis, interstitial lung disease, and antibodies directed against various synthetases of aminoacyl-transfer RNA. Other common symptoms include mechanic's hands, Raynaud's phenomenon, arthritis, and fever.

Flammer syndrome is a described clinical entity comprising a complex of clinical features caused mainly by dysregulation of the blood supply. It was previously known as vascular dysregulation. It can manifest in many symptoms, such as cold hands and feet, and is often associated with low blood pressure. In certain cases it is associated with or predisposes to the development of diseases such as a normal tension glaucoma. Flammer syndrome is named after the Swiss ophthalmologist Josef Flammer.

Cold sensitive antibodies (CSA) are antibodies sensitive to cold temperature. Some cold sensitive antibodies are pathological and can lead to blood disorder. These pathological cold sensitive antibodies include cold agglutinins, Donath–Landsteiner antibodies, and cryoglobulins which are the culprits of cold agglutinin disease, paroxysmal cold hemoglobinuria in the process of Donath–Landsteiner hemolytic anemia, and vasculitis, respectively.

Blood vessel disorder generally refers to the narrowing, hardening or enlargement of arteries and veins. It is often due to the build-up of fatty deposits in the lumen of blood vessels or infection of the vessel wall. This can occur in various locations such as coronary blood vessels, peripheral arteries and veins. The narrowed arteries would block the blood supply to different organs and tissues. In severe conditions, it may develop into more critical health problems like myocardial infarction, stroke or heart failure, which are some of the major reasons of death.

References

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